Chapter 14 Flashcards
What is the final common result of ischemia medial damage and marfans that can lead to the formation of an aneurism?
Cystic medial degeneration
Abdominal aortic aneurisms are characterized by severe atherosclerosis of the aorta, and are frequently covered by what?
How can we detect?
- Mural thrombus
- Pulsating mass in the abdomen
Aortic dissections occur when blood enters a defect in the intima and travels though the tissure between the aortic media. Who do aortic dissection most commonly occur in?
- 1. HTN males 40-60 YO
- Pts with CT disorders (Marfan)
What is the main risk factor in a aortic dissection?
HYPERTENSION
How does a patient with an aortic dissection present?
Sudden onset of severe CP that radiates to the back between the scapula and moves downward as the disscection progresses.
What is most commonly confused with a acute MI?
Aortic dissection
Who are AAA more common in?
Men
Smokers
60s
Thoracic aortic aneurism are most often due to what?
- HTN
- Marfans: Defective fibrillin and overactive TGF-B, which weakens elastic tissue
Note that we often see __________ in pts with aortic dissections.
cystic medial degeneration
- Large vessel vasculitis includes aorta and branches (2)
- Temporal (Giant cell) arteritis
- Takayasu arteritis
- Med vessel vasculitis affects muscular arteries, which supply organs
- Polyarteritis nodosa (PAN)
- Kawasaki disease
- Buerger disease
- Small vessel vasculitis affects arterioles, capillaries and venules.
- Wegener granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
Notice that __________ is the only vasculitis that involves the aorta.
giant cell arteritis
_______ requires eosinophils and is associated with asthma and atopic individuals.
Churg-Strauss
________ requires neutrophils and is associated with orogenital ulcers.
Behçet disease
_________ is associated with young male smokers.
Buerger disease
If we see a patient and ID immune complexes in the vascular wall, what do we do?
Assume DRUG HYPERSENSITIVITY!
Stop the drug => vasculitis should resolve.
Antineutrophil cytoplasmic antibodies (ANCA) are what?
autoAB against enzymes inside of cytoplasm of neutrophils, monoxytes and endothelial cells.
ANCA bind to neutrophils => activate them => cause the release of ROS and cytokines => inflame and damage endothelium => vasculitis
Do vasculitis assx with ANCA have immune complexes?
NO. ANCA produces a pausi-immune response => attack things inside of neutrophils but do not form immune comples. THUS THESE LESIONS WILL NOT HAVE IMMUNE COMPLEX DEPOSITS
PR3-ANCA/c-ANCA) target PR3 and is assx with what vasculitis?
Wegners
Anti-MPO/p-ANCA is asociated with what vasculitis?
1. Microscopic polyangiitis
2. Churg-Strauss
Measuring ANCA can tell us
how serious the disease is.
Immune complex vasculitis is seen in
- Lupus
- Drug hypersensitivity
- Secondary to exposure to infection (Hep B => Polyarteritis nodosa)
_________ are the most common causes of aortitis…
Giant Cell Arteritis and Takayasu
Non-infectious vasculitis occur how?
Immune-mediated
In what vasculitis does fibrinoid necrosis occur?
Small-cell vasculitis
- Most common form of vasculitis in older patients (>50 YO); F
Giant cell arteritis
Giant cell arteritis stems from an immune response by which cells and causes inflammation of arteries where?
- CD4+
- Proximal aorta, carotid artery and branches
Histo in Giant cell arteritis
- CD4+ cells are going to infiltrate the internal elastic lamina and the media.
- MO invate the media and form multinucleated giant cells inside of a granuloma.
- Fragmented ILA and intimal thickening => narrow lumen and ischemia
Inflammation in Giant cell arteritis is what?
Patchy and segmental
Sx in giant cell arteritis
- Temporal artery: HA, facial pain on temporal area
- JAw claudication
- Opthalmic artery: visual disturbacne and double vision
Takayasu arteritis most commonly affects who
Japanese (AZN) females under 40.
Takayasu Arteritis (aka pulseless disease)
Granuloma vasculitis of medium and large arteries characterized by ocular disturbances and marked weak pulses/ low BP in the UE’s
Takayasu arteritis has similar histological findings as those seen in giant cell arteritis (T-cells infiltrate of media and intimal elastic lamina, granulomas and intimal thickening, patchy and focal), except involves which vessels?
Medium and large vessels: Aortic arch and nearby (pulmonary (1/2), carotid and renal); NOT TEMPORAL
Since many features of Takayasu arteritis are shared with Giant Cell arteritis, the distinction between the 2 is primarily made how?
- AGE of the pt
- Takayasu = younger(<50 yo)
- Giant cell = older (>50 yo)
Sx in Takayasi
- Initial sx are non-specific (fatigue, WL, fever, etc)
- Later
-
Vascular symptoms: “pulseless disease” because the lumen of brachiocephalic / carotid / subclavian arteries narrows => decrease BP => weak/absent UE/carotid pulses
- Bruits form d/t turbulent blood flow
- Vascular symptoms then go down to the abdominal aorta and distal aorta
- Pulmonary HTN
- MI
- Renal HTN
- Limb claudication (pain w walking that is relieved w rest
- Ocular disturbances: visual disturbances, retinal hemorrhage, blindness
-
Vascular symptoms: “pulseless disease” because the lumen of brachiocephalic / carotid / subclavian arteries narrows => decrease BP => weak/absent UE/carotid pulses
.
Polyarteritis Nodosa (PAN) is what
fibrinoid necrosis that involves multiple medium sized arteries and involves many organs (SPEC RENAL), but NOT the lungs (pulmonary vessels).
PAN most commonly affects the _____, but not the _____
MC: KIDNEYS
NOT: lungs
In 1/3 of pt’s with polyarteritis nodosa the vasculitis is attributable to chronic infection by which virus?
Immune-complexes composed of what?
HBsAg and anti-HBsAg = Chronic HBV
What process does PAN cause vasculitis
most likely immune complex mediated
List the 4 vessels most often involved in Polyarteritis Nodosa in descending order of frequency.
- - Kidneys
- - Heart
- - Liver
- - GI tract
Histo of PAN
- Polyarteritis nodosa results in a beads on a string appearance that are not circumferential. Because PAN causes acute flares and relapses , it makes lesions at varying stages.
- Early lesions are segmental, transmural (occur across the entire wall= PAN) necrotizing inflammation at BRANCH POINTS (NO GRANULOMAS)
- Often time we can see immune complexes in wall of BV.
- As the wall weakens, they can create an aneurism.
- Lesions that heal => fibrosis, creating the beads on a string.
- Early lesions are segmental, transmural (occur across the entire wall= PAN) necrotizing inflammation at BRANCH POINTS (NO GRANULOMAS)
In PAN are sites of inflammation circumferential
NO
Classic Polyarteritis Nodosa is characterized by what morphological pattern of inflammation?
Segmental TRANMURALnecrotizing inflammation of small- to medium-sized arteries that can heal and fibrose
During acute phase of Polyarteritis Nodosa the transmural inflammation of arterial wall shows a mixed inflammatory infiltrate and is often accompanied by what type of necrosis?
Fibrinoid necrosis
Polyarteritis Nodosa is typically a disease affecting which age group?
Typical course of the disease?
- Young
- Remitting and episodic
Does Polyartertis nodosa form granulomas?
NO
What is a “classic” presentation (signs/sx’s) of Polyarteritis Nodosa?
1. Rapidly accelerating HTN
2. Abdominal pain and bloody stools
3. Myalgias and peripheral neutitis
Tx of PAN
immunosupression; because immune complex mediated
Kawasaki disease
who does it affect
Acute arteritis of the coronary arteries (medium) that affect kids and infants under 4
Clinical significance of Kawasaki Disease stems from its predilection for which artery?
Leading to what?
Coronary artery
Aneurisms => thrombosis or rupture => <strong>MI</strong>
What is the morphology of the vasculitis seen in Kawasaki Disease; differs from PAN how?
Dense transmural inflammatory infiltrate w/ LESS prominent fibrinoid necrosis than in PAN
Kawasaki disease typically presents with what signs/sx’s?
*First Aid Mnemonic
CRASH and burn
- Conjunctival injection
- Rash (polymorphous -> desquamating)
- Adenopathy (cervical LN enlargement = mucocutaneous LN syndrome)
- Strawberry tongue (erythema and blistering of oral mucosa)
- Hand and feet are red
- Burn = FEVER for longer than 5 days
If Kawaksaki Disease is recognized early in its course treatment with what will sharply reduce the risk of symptomatic coronary artery disease?
IV immunoglobulin and Aspirin
Microscopic polyangiitis generally affects which type of vessels?
Capillaries + small arterioles and venules
How do the age and distribution of the lesions seen in Microscopic Polyangiitis differ from PAN?
Tend to be of the same age and are distributed more widely
Microscopic polyangiitis affects vessels of many organ systems, but which 3 systems are most common?
- -Kidneys (90% have necrotizing glomerulonephritis)
- -Lungs (pulmonary hemorrhage)
- -Skin (palpalble pupura
*
What are other names for micrscopic polyangittis
- Hypersensitivity vasculitis
- leukocytoclastic vasculitis
MPA can be features of what 3 disorders
- Henoch-Schonlein purpura
- Essential mixed cryoglobulinemia
- Vasculitis assoc. w/ CT disorders
What is seen on histo of MPA?
- P-ANCA will be destroying neutrophils (thus, many fragmented PMNs) => cause segmental fibrinoid necrosis
Does MPA form granulomas?
NO
Which immune cells are seen infiltrating in microscopic polyangiitis, many of which are undergoing apoptosis and giving rise to the term leukocytoclastic vasculitis?
NEutrophils (Fragment PMN)
What are the major clinical features (signs/sx’s) seen with microscopic polyangiitis?
- Hemoptysis (cough up blood)
- Hematuria and Proteinuria (pee blood/proteins)
- Bowel pain or bleeding
- Muscle pain or weakness
- Palpable cutaneous purpura
What vasculitis is a pulmonary-renal syndrome?
MPA
What does MPA stain + for and and what other vasculitis has palpable purpure
- p-ANCA
- Churgg-Strauss
Q: How is MPA different from PAN?
- Small BV; lesions are same age and more widely distributed
What is Churgg Straus disease often misdx with?
1. Allergies
2. Allergici rhinitis
3. Asthma
CHURGG!
What is Churgg Strauss disease?
small-vessel necrotizing vasculitis that involves mainly the heart and lungs. !!!
Churgg-Strauss syndrome may have inflammation that resembles PAN or microscopic polyangiitis with the addition of what 2 things?
1. HIGH AMOUNT OF EOSINOPHILS
2. NECROTIZING GRANULOMAS
What is the primary manifestation of Churg-Strauss Syndrome affecting the kidneys?
Focal and segmental glomerulosclerosis
What is the primary manifestation of Churg-Strauss Syndrome affecting the myocardium?
Clinically significant why?
Cardiomyopthy: MCC of death in this
- Cutanous involvement of Churg-Strauss Syndrome presents with what?
Palpalpe purpura, also seen in MPA
Behçet Disease is a vasculitis that affects small-medium vessels that presents w what triat?1
1. Oral ulcers
2. Genital ulcers
3. Uveitis
Which HLA haplotype is associated with Behcet Disease?
HLA-B51
Vessel inflammation in Behcet Disease is due to what inflammatory cell type?
Recruited by which T cells?
Neutrophils, recruitsed by TH17 cells
Mortality seen in Behcet Disease is related to what 2 complications?
1. Neruo involvemtn
2. Rupture of aneurism
MPO-ANCA’s (P-ANCA) are found in what 2 types of non-infectious vasculitis?
- MPA
- Churgg Strauss
C-ANCA (PR3) is assx with
weCners
Granulomatosis with Polyangitis (Wegener granulomatosis) is a c-ANCA (PR3-ANCA) + necrotizing vasculitis characterized by a triad of what?
- 1) Necrotizing granulomas of upper or lower respiratory tract, or both
2) Necrotizing or granulomatous vasculitis most prominenetly in lungs
3) Focal necrotizing, often CRESCENTERIC, glomerulonephritis
Granulomatosis with polyangiitis is a form of hypersensitivity mediated by what?
- Inhalation of airborne toxin => T-cell mediated hypersensitivy => develops c-ANCA (PR3-ANCA) =>
Which sex is more often affected by granulomatosis with polyangiitis and at what age?
men 40 YO
What are the classic features (signs/sx’s) of granulomatosis with polyangiitis? (4)
- Persistent pneumonitis w/ bilateral nodular and cavitary infiltrates
- - Chronic sinusitis
- Nasopharyngeal ulcerations
- Evidence of renal disease
How serious is granulomatosis with polyangiitis if left untreated?
rapidly; 80% will die
which vascilitis has
granulomas with GEOGRAPHIC patterns of central necrosis?
WEGNERS (GPA)
What may be seen radiographically in the lower respiratory tract of pt w/ granulomatosis with polyangiitis as multiple necrotizing granulomas coalesce?
centrally cavitating lesions
Which renal lesions may be seen early on in granulomatosis with polyangiitis?
Eventually progress to what in late stages?
- Focal and segmental glomerulonephritis
- Crescenteric glomerulonephritis
Thromboangiitis Obliterans (Buerger Disease) is STRONGLY assx with tobacco smoke in ppl YOUNGER 35 YO and affects TIBIAL and RADIAL As
- Israeli, Indian, and Japanese groups who have a hypersensitivity to tobacco products.
-
Segmental thrombosis w/ microabcesses formed => total obliteration of distal vessel lumens => distal ischemia of the of distal extremities
- Tibial and radial arteries=> loss of the fingers and toes.
- Inflammation of affected vessels can spread to veins and nerves => neuropathy =>
- Cold-induced Raynaud phenomenon
- Intermittent claudication (pain with XRCISE that stops with rest)
- Superficial phlebitis (venous inflammation)
- Ulcers and gangrene of fingers and toes.
- Causes severe pain, even at rest.
- Histo:
- Segmental, granuloma formation with thrombus that may have microabsesses that obliterates lumen of vessel.
Which 3 organisms can directly invade vessels and cause infectious vasculitis?
- Pseudomonas
- Aspergillus
- Mucor
Infectious vasculitis usually spreads how?
· Infection of local tissue, however, they can spread through the blood too.
Vascular infections (infective vasculitis) can weaken arterial walls and can result in what clinically significant events?
· Mycotic aneurysms or cause thrombosis and infarction
What is Raynaud’s phenomenon?
Excessive vasospasm (RED, WHITE and BLUE) of small arteries and arterioles, in the fingers and toes: proximal vasodilation, central vasoconstriction and distal cyanosis
How does Primary Raynaud’s vs. Secondary differ in their symmetry of involvement of the digits?
Primary has symmetrical involvement of the digits and is not associated with an underlying disease. It is caused cold or emotion.
· Most common in young F, benign
- Secondary has asymmetrical involvement of the digits and is assx with a underlying condition: SLE, scleroderma. Gets worse w time.
Excessive vasoconstriction of myocardial arteries or arterioles which may cause ischemia or infarct is known as what?
· Cardiac Raynaud
What are endogenous and exogenous agnts that can prolonged myocardial vessel contraction?
- Endogenous: EPI or a pheochromacytoma
- Exogenous: cocaine
Cardiac Raynaud can result in what?
- Sudden cardiac death
- takotsubo cardiomyopathy “broken <3” syndrome d/t emotional distress
Varicose veins are abnormally dilated, tortous veines caused by prolonged, increased intraluminal pressure and most often affect which veins?
Superficial veins or the upper and lower legs.
What are complications of varicose veins?
- Stasis
- congestion,
- stasis dermatitis (pain and ischemia on overlying skin),
- poor wound healing,
- ulcers
How frequent do emboli arise from superficial LE veins?
RARE, compared to deep LE veins (DVT).
What can cause esophageal varices and why are they clinically important?
- Portal HTN: opens shunt and sends blood to veins at GE junction: they can rupture and kill.
What are hemorrhoids
Dilation of venous plexus at anorectal junction
What is thrombophlebitis and where does it most commonly occur?
inflammatory process that causes blood clot to form and block a VEIN, usually in your leg (forms DVT)
What is the most serious potential complication associated with DVT’s and is often the first manifestation of thrombophlebitis
What are the most important risk factors for developing a DVT?
- PE
- prlong activity/not moving; hypercoagulbility
What is migratory thrombophlebitis (Trousseau syndrome)?
MCC in?
- A clot that moves around the body: appears at one site, disappears, then goes to another.
- Mucin secreting ADENOCARCINOMAS, often a paraneoplastic syndrome
What are the major causes of Superior Vena Cava Syndrome?
neoplasms
aortic aneurysms
What is Superior vena cava syndrome
pressure on the SVC is due to a neoplasm compressing it => leads to cyanosis and dilation of the veins of the head, neck and arms and can cause respiratory distress if pulmonary vessels are compressed
What is inferior vena cava syndrome
neoplasma compress IVC or thrombosis of hepatic, renal or LE veins that go up =>
- LE edema,
- distension of superficial collateral vein in the lower abdomen.
- If the kidneys are involved => massive proteinuria.
Which 2 cancers have a tendency to grow within veins and may ultimately lead to inferior vena cava syndrome?
May also be caused by thrombosis from where?
- - Hepatocellular carcinoma
- - Renal cell carcinoma
- - Thrombosis of the hepatic, renal, or LE veins which propogate cephalad
Most common agent of lymphangitis is
Group A B-hemolytic Strep
;/hat is lymphangitis and what most commonly causes it?
- acute inflammation and the spread of bacterial infection into the lymphatics => red painful subcutaneous streats and painful enlargement of draining LN
What is lymphadenitis
painful enlargment of draining LN
_______ lymphedema is congenital, either an isolated defect or “familial Milroy disease,” which is lymphatic agenesis
Primary
____ lymphedema is a blockage of previously normal lymphatics d/y tumor, etx
secondary
peau d’ orange is an example of what
secondary (obstructive lymphedema)
- Tumor cells block draining lymphatic from breast cancer, causing the skin over it to look like ORANG PEELS
local dilation of a structure
Ectasia
A permanent dilation of a small vessels that forms a red lesion in the skin or mucous membrane. They can be congenital or acquired. What are they called and are they true neoplasms?
- Telangiectasia; no
Nevus flammeus
: a vascular ectasia that forms a light pink-purple birthmark on the head or neck d/t dilated vessels. They often regress by themselves
How do Cavernous Hemangiomas differ in terms of infiltration and regression as compared to Capillary Hemangiomas?
- Are more infiltrative and frequently involve deep structures
- Do NOT spontaneously regress
- Locally destructive!
_______ are very common tumors characterized by increased numbers of normal or abnormal BV
hemangiomas
Congenital (juvenile/strawberry) hemangiomas often what…
REGRESS
What is a capillary hemangioma
most common; thin wall capillaries are tightly packed 2gether
what is this
Cavernous hemangiomas: irregular, dilated BV form a lesion with a irregular border, often involving deep tissue and are more likely to bleed
____________ (lobular capillary hemangioma) are actually rapidly-growing hemangiomas that exist the oral mucosa and can ulcerate.
When do they commonly occur?
Pyogenic granuloma
Granuloma gravidarium (pregnany tumor:
What is a pyogenic granuloma
A CAPILLAR HEMANGIOMA!
not a pyogenic granuloma
